{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T04:25:23Z","timestamp":1789619123823,"version":"build-2803163510"},"reference-count":41,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2023,2,21]],"date-time":"2023-02-21T00:00:00Z","timestamp":1676937600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Genes Chromosomes &amp; Cancer"],"published-print":{"date-parts":[[2023,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Although well known as a fusion partner in hematological malignancies, fusion genes involving the ABL proto\u2010oncogene 1 (<jats:italic>ABL1<\/jats:italic>), mapping to chromosomal region 9q34.12, have only been anecdotally reported in five soft tissue tumors. These neoplasms have been variously reported as perineurioma, angiofibroma, and solitary fibrous tumor, and all have harbored a <jats:italic>GAB1::ABL1<\/jats:italic> gene fusion; however, the nosology and clinicopathological characteristics of soft tissue tumors carrying this rare fusion have not been delineated. We herein describe eight tumors containing the <jats:italic>GAB1::ABL1<\/jats:italic> fusion and review previously reported cases in a series to define their morphological spectrum, address immunohistochemical evidence for a line of differentiation, with special reference to the presence or absence of a perineurial immunophenotype, and gather insight into their behavior. The patients included four females and four males, aged 13\u201337\u2009years (median, 24\u2009years). Two cases each originated in the shoulder area, trunk, hands, and lower extremities, with a size range of 1.5\u20138\u00a0cm (median, 3.4\u00a0cm). Four tumors were deep and four superficial. All tumors were morphologically similar, being composed of bland fibroblast\u2010like spindle to ovoid cells diffusely arranged in a paucivascular fibrous to fibromyxoid stroma with variable resemblance to soft tissue perineurioma. Mitotic activity was generally low (0\u20138 mitoses in 10 high\u2010power fields [HPFs]; median, 1). All lesions had at least focally infiltrative margins, but they otherwise lacked pleomorphism and necrosis. Immunohistochemistry showed focal reactivity for CD34 (5\/7), epithelial membrane antigen (EMA) (3\/8), claudin1 (2\/3), GLUT1 (4\/6), and S100 (2\/7); other markers, including MUC4 (0\/7), desmin (0\/9), and smooth muscle actin\u00a0(SMA) (0\/4), were negative. RNA sequencing revealed a <jats:italic>GAB1::ABL1<\/jats:italic> fusion in all cases with exon 6 of <jats:italic>GAB1<\/jats:italic> fused to exon 2 of <jats:italic>ABL1<\/jats:italic>. Treatments included various forms of surgical intervention in seven cases; one tumor was biopsied only. Limited follow\u2010up was available for five patients. One tumor regrew rapidly within 1\u2009month to 1.5\u00a0cm after an initial marginal excision and was re\u2010excised with close margins. Four patients were disease\u2010free at 1, 3, 14, and 25\u2009months of follow\u2010up. Metastases have not, to date, been observed. This series characterizes \u201c<jats:italic>GAB1::ABL1<\/jats:italic> fusion\u2010positive spindle cell neoplasm\u201d as a distinct entity, with overlapping features with soft tissue perineurioma and predilection for children and young adults.<\/jats:p>","DOI":"10.1002\/gcc.23131","type":"journal-article","created":{"date-parts":[[2023,2,6]],"date-time":"2023-02-06T13:48:32Z","timestamp":1675691312000},"page":"449-459","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["<i>GAB1::ABL1<\/i> fusions define a distinctive soft tissue neoplasm, with variable perineurial differentiation, and a predilection for children and young adults"],"prefix":"10.1002","volume":"62","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0445-8161","authenticated-orcid":false,"given":"Abbas","family":"Agaimy","sequence":"first","affiliation":[{"name":"Institute of Pathology, Erlangen University Hospital, Comprehensive Cancer Center, European Metropolitan Area Erlangen\u2010Nuremberg (CCC ER\u2010EMN) Friedrich Alexander University of Erlangen\u2010Nuremberg  Erlangen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raul","family":"Perret","sequence":"additional","affiliation":[{"name":"Department of Biopathology Institut Bergoni\u00e9, Comprehensive Cancer Center  Bordeaux France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth G.","family":"Demicco","sequence":"additional","affiliation":[{"name":"Department of Pathology &amp; Laboratory Medicine Mount Sinai Hospital  Toronto Ontario Canada"},{"name":"Department of Laboratory Medicine and Pathobiology University of Toronto  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4444-0297","authenticated-orcid":false,"given":"John","family":"Gross","sequence":"additional","affiliation":[{"name":"Department of Pathology The Johns Hopkins Hospital  Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6040-3109","authenticated-orcid":false,"given":"Yajuan J.","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology, Clinical Genomics Laboratory University of Washington School of Medicine  Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rihab","family":"Azmani","sequence":"additional","affiliation":[{"name":"Bioinformatics, Data and Digital Health Department Institut Bergoni\u00e9, Comprehensive Cancer Center  Bordeaux France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carsten","family":"Engelmann","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery Brandenburg Clinic Center  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Schubart","sequence":"additional","affiliation":[{"name":"Institute of Pathology, Erlangen University Hospital, Comprehensive Cancer Center, European Metropolitan Area Erlangen\u2010Nuremberg (CCC ER\u2010EMN) Friedrich Alexander University of Erlangen\u2010Nuremberg  Erlangen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joosep","family":"Seppet","sequence":"additional","affiliation":[{"name":"Pathology Department Tartu University Hospital  Tartu Estonia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Stoehr","sequence":"additional","affiliation":[{"name":"Institute of Pathology, Erlangen University Hospital, Comprehensive Cancer Center, European Metropolitan Area Erlangen\u2010Nuremberg (CCC ER\u2010EMN) Friedrich Alexander University of Erlangen\u2010Nuremberg  Erlangen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fran\u00e7ois","family":"Le Loarer","sequence":"additional","affiliation":[{"name":"Department of Biopathology Institut Bergoni\u00e9, Comprehensive Cancer Center  Bordeaux France"},{"name":"Bordeaux Institute of Oncology, BRIC U1312, INSERM, Universit\u00e9 de Bordeaux Institut Bergoni\u00e9  Bordeaux France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2269-6216","authenticated-orcid":false,"given":"Brendan C.","family":"Dickson","sequence":"additional","affiliation":[{"name":"Department of Pathology &amp; Laboratory Medicine Mount Sinai Hospital  Toronto Ontario Canada"},{"name":"Department of Laboratory Medicine and Pathobiology University of Toronto  Toronto Ontario 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